Hirokawa Kazuhiko, Sato Shunichi, Hiraoka Eiji, Iwanami Keiichi
Department of Internal Medicine, Tokyo Bay Urayasu Ichikawa Medical Center, Chiba, Japan.
Department of Rheumatology, Tokyo Bay Urayasu Ichikawa Medical Center, Chiba, Japan.
Mod Rheumatol Case Rep. 2025 Jul 25;9(2). doi: 10.1093/mrcr/rxae087.
Diffuse alveolar haemorrhage (DAH) is a rare and severe complication of IgA vasculitis (IgAV). Although glucocorticoids and immunosuppressive agents are used for its treatment, there is no consensus on the optimal form of treatment. We herein report the case of a 53-year-old, female patient with IgAV. She was initially resistant to glucocorticoid therapy and experienced acute respiratory failure due to DAH but responded well to rituximab (RTX) and plasma exchange (PLEX). While some previous case reports have suggested that RTX or PLEX can be effective for severe IgAV, there are no reports of a combination of RTX and PLEX being used successfully to treat IgAV-associated DAH. In the model of IgAV pathogenesis proposed herein, aberrant IgA1 and IgA-specific IgG autoantibodies play a pivotal role. PLEX may facilitate the prompt removal of these circulating, aberrant immunoglobulins while RTX inhibits their further production. Consequently, a combination of RTX and PLEX may represent an effective treatment approach for severe glucocorticoid-refractory cases of IgAV.
弥漫性肺泡出血（DAH）是IgA血管炎（IgAV）一种罕见且严重的并发症。尽管糖皮质激素和免疫抑制剂用于其治疗，但对于最佳治疗方式尚无共识。我们在此报告一例53岁IgAV女性患者的病例。她最初对糖皮质激素治疗耐药，因DAH出现急性呼吸衰竭，但对利妥昔单抗（RTX）和血浆置换（PLEX）反应良好。虽然之前一些病例报告提示RTX或PLEX对重症IgAV可能有效，但尚无RTX联合PLEX成功用于治疗IgAV相关DAH的报告。在本文提出的IgAV发病机制模型中，异常IgA1和IgA特异性IgG自身抗体起关键作用。PLEX可能有助于迅速清除这些循环中的异常免疫球蛋白，而RTX则抑制其进一步产生。因此，RTX联合PLEX可能是重症糖皮质激素难治性IgAV病例的一种有效治疗方法。
